Biochemical and ultrastructural study of neurofibrillary tangles in amyotrophic lateral sclerosis/parkinsonism-dementia complex in the Kii peninsula of Japan

Nobuo Itoh, Koichi Ishiguro, Hiroyuki Arai, Yasumasa Kokubo, Ryogen Sasaki, Yugo Narita, Shigeki Kuzuhara

Research output: Contribution to journalArticlepeer-review

21 Citations (Scopus)

Abstract

Amyotrophic lateral sclerosis/parkinsonism-dementia complex of the Kii peninsula (Kii ALS/PDC) is a neuro-degenerative disorder endemic to natives in the southern coast area of the Kii peninsula of Japan. The disorder closely resembles Guamanian ALS/PDC clinically and neuropathologically. The characteristic neuropathological finding is abundant neurofibrillary tangles (NFTs) without amyloid deposition. To elucidate the biochemical properties of hyperphosphorylated tau protein, the major component of the NFTs, we examined Kii ALS/PDC brains by immunoblotting and immunohistochemical analysis using well-characterized anti-tau antibodies specific to phosphorylation-dependent or -independent epitopes. Hyperphosphorylated tau in Kii ALS/PDC had phosphorylated epitopes common to tau of paired helical filaments (PHFs) in Alzheimer disease (AD); immunoblot showed triplet bands composed of 6 tau isoforms. Ultrastructurally, NFTs revealed a twisted filamentous shape similar to PHF of AD. The biochemical properties of its phosphorylated tau protein and the ultrastructural characteristics of the NFTs of Kii ALS/PDC are very similar, if not identical, to PHF tau in AD, although they are different taupopathies.

Original languageEnglish
Pages (from-to)791-798
Number of pages8
JournalJournal of Neuropathology and Experimental Neurology
Volume62
Issue number7
DOIs
Publication statusPublished - 2003 Jul 1

Keywords

  • Amyotrophic lateral sclerosis/Parkinsonism-dementia complex
  • Kii peninsula
  • Neurofibrillary tangle
  • Paired helical filaments
  • Tau protein

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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